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Typical Kawasaki disease
Italian Journal of Pediatrics volume 41, Article number: A47 (2015)
Kawasaki disease (KD) was first described in Japan in 1967 by Tomisaku Kawasaki . It is an acute, systemic, self-limited vasculitis, whose targets are medium diameter vessels in each organ and apparatus. It is a vasculitis of unknown aetiology, probably multifactorial, that occurs predominantly in infants and young children. The most important complications are coronary arteries aneurysms or ectasia, that develop in 15% to 25% of untreated patients, but only in 5% of patients treated with immunoglobulins within ten days following onset of fever [2, 3].
Typical Kawasaki disease is characterized by signs and symptoms which are defined as “diagnostic clinical criteria”, and are:
Diagnosis of KD is based on clinical criteria [3, 4]: there are neither typical clinical features nor diagnostic tests. An increasing number of children is reported in literature, having coronary artery aneurysms or ectasia, but who do not fulfill all diagnostic criteria. These cases are defined as incomplete KD and atypical KD.
Typical KD is diagnosed if:
fever = 5 days associated with = 4 diagnostic criteria, even before performing echocardiography
fever = 5 days associated with < 4 diagnostic criteria and possible coronary arteries lesions
fever at fourth day with = 4 diagnostic criteria and possible coronary arteries lesions.
Finally, other clinical findings may be present in KD:
Cardiac: coronaritis, pericarditis, myocarditis, endocarditis, mitral regurgitation, aortic and tricuspidal regurgitation (in the acute phase), aortic dilatation (in the later phase), cardiac failure, shock, arrhythmia, coronary artery involvement (in the sub acute phase)
Vascular: Raynaud phenomenon, peripheral gangrene
Joints: arthritis or arthralgia
Nervous System: irritability, aseptic meningitis, sensor-neural hearing loss, transient unilateral peripheral facial nerve palsy
Gastrointestinal: diarrhoea, vomiting, abdominal pain, acute surgical abdomen, hepatic enlargement and jaundice, acute acalculous distention of the gallbladder (hydrops)
Urinary: aseptic pyuria, urethritis, testicular swelling
Skin: erythema and induration at the site of a previous vaccination with Bacille Calmette-Guérin, Beau lines
Respiratory: cough, rhinorrea, pulmonary nodules and infiltrates.
Kawasaki T: Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children. Aerugi. 1967, 16 (3): 178-222.
Kato H, Sugimura T, Akagi T, Kato H, Sugimura T, Akagi T, et al: Long term consequences of Kawasaki disease. A 10- to 21-year follow-up study of 594 patients. Circulation. 1996, 94 (6): 1379-1385. 10.1161/01.CIR.94.6.1379.
Dajani AS, Taubert KA, Gerber MA, Shulman ST, Ferrieri P, Freed M, et al: Diagnosis and therapy of Kawasaki disease in children. Circulation. 1993, 87 (5): 1776-1780. 10.1161/01.CIR.87.5.1776.
Newburger JW, Takahashi M, Gerber MA, Gewitz MH, Tani LY, Burns JC, et al: Diagnosis, treatment, and long-term management in Kawasaki disease: a statement for health professionals from the committee on rheumatic fever, endocarditis and Kawasaki disease, council on cardiovascular disease in the young, American Heart Association. Pediatrics. 2004, 114 (6): 1708-1733. 10.1542/peds.2004-2182.
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Marchesi, A., Tarissi, I., Marucci, G. et al. Typical Kawasaki disease. Ital J Pediatr 41, A47 (2015). https://doi.org/10.1186/1824-7288-41-S2-A47
- Kawasaki Disease
- Aseptic Meningitis
- Coronary Artery Aneurysm